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在XTEND-Kids 3期多国试验中,对年龄<12岁、接受每周一次依奈索托考因子α预防治疗的重度甲型血友病儿童的出血事件及临床相关药代动力学参数进行事后分析。

Post hoc analysis of bleeding episodes and clinically relevant pharmacokinetic parameters among children <12 years old with severe hemophilia A receiving once-weekly efanesoctocog alfa prophylaxis in the XTEND-Kids phase 3 multinational trial.

作者信息

Malec Lynn, Mathias Mary, Dunn Amy L, Nolan Beatrice, Wong Nancy, Bystrická Linda, Khan Umer, Neill Graham, Santagostino Elena, Gunawardena Sriya, Tarango Cristina, Peyvandi Flora

机构信息

Versiti Blood Research Institute, Milwaukee, Wisconsin, USA; Departments of Medicine and Pediatrics, Medical College of Wisconsin, Milwaukee, Wisconsin, USA.

Great Ormond Street Hospital for Children National Health Service Foundation Trust, London, UK.

出版信息

J Thromb Haemost. 2025 Jul 21. doi: 10.1016/j.jtha.2025.07.011.

Abstract

BACKGROUND

Once-weekly efanesoctocog alfa (50 IU/kg) provided high-sustained factor (F)VIII activity and robust bleed protection in previously treated children with severe hemophilia A.

OBJECTIVES

This post hoc analysis presents additional bleed treatment and pharmacokinetic (PK) outcomes from the XTEND-Kids study (NCT04759131).

METHODS

XTEND-Kids was a phase 3, open-label study in previously treated children aged <12 years with severe hemophilia A. Participants received once-weekly efanesoctocog alfa (50 IU/kg) prophylaxis for 52 weeks. Annualized bleed rate, bleed treatment, response to treatment, PK, and safety outcomes, including immunogenicity, were assessed.

RESULTS

Once-weekly prophylaxis with efanesoctocog alfa (50 IU/kg) provided high-sustained FVIII levels in the normal to near-normal range (>40 IU/dL) for 3 days and >10 IU/dL for approximately 7 days at steady state; PK parameters were similar in participants with blood groups O and non-O. Efanesoctocog alfa prophylaxis resulted in effective bleed protection with zero bleeds in 64% of participants and consistently low annualized bleed rates. The rate of spontaneous and traumatic bleeding episodes per week remained consistently low throughout the 52-week study period. A single dose of efanesoctocog alfa resolved 95% of bleeding episodes, regardless of type or location. The hemostatic efficacy of efanesoctocog alfa for the treatment of bleeding episodes was rated as excellent or good for nearly all evaluated first injections (97%). No inhibitors or unexpected safety findings were reported.

CONCLUSION

Once-weekly prophylaxis with efanesoctocog alfa provided high-sustained FVIII levels and highly effective bleed treatment and prevention in previously treated children aged <12 years with severe hemophilia A.

摘要

背景

每周一次的依那西普α(50 IU/kg)在既往接受治疗的重度甲型血友病儿童中可提供持续的高因子(F)VIII活性及有力的出血防护。

目的

本事后分析展示了XTEND-Kids研究(NCT04759131)的额外出血治疗及药代动力学(PK)结果。

方法

XTEND-Kids是一项针对既往接受治疗的12岁以下重度甲型血友病儿童的3期开放标签研究。参与者接受每周一次的依那西普α(50 IU/kg)预防治疗,为期52周。评估年化出血率、出血治疗情况、治疗反应、PK及安全性结果,包括免疫原性。

结果

每周一次的依那西普α(50 IU/kg)预防治疗在稳态时可使F VIII水平在正常至接近正常范围(>40 IU/dL)持续3天,>10 IU/dL持续约7天;O型血和非O型血参与者的PK参数相似。依那西普α预防治疗可有效预防出血,64%的参与者无出血情况,年化出血率持续较低。在整个52周的研究期间,每周的自发性和创伤性出血事件发生率一直较低。单剂量依那西普α可解决95%的出血事件,无论出血类型或部位如何。对于几乎所有评估的首次注射,依那西普α治疗出血事件的止血效果被评为优秀或良好(97%)。未报告有抑制剂或意外的安全性发现。

结论

每周一次的依那西普α预防治疗可在既往接受治疗的12岁以下重度甲型血友病儿童中提供持续的高F VIII水平以及高效的出血治疗和预防。

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